Merry Christmas from the Willms' Family! Happy New Year too!
Emmett loves Christmas. It is a time of wonder. A time of colour and a bit of extravagance. A time to talk about Baby Jesus. The tree is awesome and when it first went up he had mom, dad, or whoever was close by pull the decorations off one by one so that he could touch them and then get them back on the tree. And then he went through the ritual again! He loves looking at nativity scenes and is especially intrigued by the baby Jesus. Presents ... we'll have to get back to you on that because we don't know yet. Pam got an idea from the internet about using ribbons as something for him to grab onto and rip open the present. Great idea - we'll have to see how it works. And in keeping with Emmett's needs, I used a tenth of the scotch tape that I would normally use to seal up presents. Emmett is still not sure about the snow, however, and while he does eventually warm up to it, his first reaction is that of tears.
Reasons for not liking the snow? First of all, it's cold. All little kids are affected by the cold more than adults. Then you bundle them all up and they can barely move ... well, bundle Emmett up and guaranteed he cannot move! Second, power wheelchairs don't travel well in snow. Makes sense, but it also means that Emmett's movements are limited. Here in beautiful BC we're now into the third week of snow - unexpected and unusual. Third, a combination of the first two - you cannot fit into a wheelchair all bundled up. When getting strapped into the car bed for transportation all bulky outerwear is to be removed - meaning you get a shock of cold. Three good reasons for Emmett not to like snow. The best reason for this pic is the little tear that is rolling down from Emmett's eye! But don't worry, he was laughing soon afterwards. By the way, Abbotsford is now into the third week of having snow. Locals are in shock. Right now it looks like it will be a white Christmas.
Emmett is surrounded by people who love and support him - and want him to get into the Christmas spirit. But Emmett does not like weird things on his head! Give him a jaunty cap and he'll flaunt it everywhere. Put a Santa hat on him and the faces he makes are awesome in revealing his distress. This little boy is a clothes horse whose fashion sense dictates what naughty or nice ... and a Santa hat is pure distress! I wonder what he would do if he were to meet Santa Claus! We won't find that out this year - too many germs hanging out around Santa Claus displays so they will get a wide berth!
In an earlier post we commented how Emmett not only loved his Christmas tree, but that he was fascinated by nativity scenes. Here at Grandma's house he was taking his chair closer and closer to her Charlie Brown's Christmas nativity scene. Then he'd wander off and explore the house, but then back to the nativity scene. I think Alisha and Josh were worried that he was going to knock them over, so Josh picked up the pieces and let Emmett get a closer look. Snoopy dressed up as a lamb is really pretty cool! It is very interesting to note that last year Emmett's favourite Christmas toy was a feather (a bunch of feathers, actually) because it was easy for him to pick up and play with. This year Emmett only grabs a feather to tease Wendell and, when Wendell steals the feather, he cries. His favourite toys are toy cars and airplanes that he loves to pick up. He has even held Baby Jesus from the nativity set (I'm not sure if Grandma knows that!). His head control is so much better and his head is rarely resting on his chair's head support.
Of course, it isn't truly a family gathering without scaring a little one. Purposely! Just like knowing that a Santa hat will cause grief, we have all learned that while Emmett loves a partially deflated giant Nemo the clownfish balloon the opposite is also true. When Nemo is newly filled he causes Emmett slight consternation. When Nemo is activated and swimming around the house, then Emmett fusses and panics. In his chair he will try to get away. Of course, if his mom is driving Nemo/swimming Nemo/flying Nemo (not that I'm saying that Alisha is purposefully chasing her son's wheelchair with a giant, remote-controlled clownfish that she knows causes him to be afraid) there's not a place of safety anywhere until Grandpa takes the remote away from her! And threatens her with a time out!
Emmett loves Wendell, his early Christmas present. They are absolutely best friends - at least, as long as Wendell doesn't invade Emmett's space and control his private property. As Wendell is a cat, you know that he views other's private sanctuaries as his own. Battles result.
Of course, a Christmas blog wouldn't be complete without a video of Emmett in water showing off his moves. Of course, while he will usually spin around in multiple 360° circles - as he knew the camera was on there was not as much action as normal. Emmett was recently at the public pool, and he just floated. He was mesmerized by all of the sounds, lights, and actions of other swimmers.
The USA Food and Drug Administration APPROVED Spinraza on Friday, December 23, 2016. This is the amazing drug that Emmett has been receiving (it has had three names since we first heard about it) and now, when a little one is diagnosed with Spinal Muscular Atrophy their parents will be given an option for medical treatment. This is huge and it is the first drug approved to fight SMA! What an amazing Christmas present!
On our journey of finding out more about this genetic disease called Spinal Muscular Atrophy, we have found on FaceBook and other sources inspirational success stories of youngsters who have beaten the odds and are in University - and that is without the amazing drug, Nusinersen, that Emmett is receiving. At the same time, our journey has placed us beside others who have little ones have not been able to survive this disease which literally shuts down the nerves in the spinal column and causes all muscles to atrophy (stop working). That's bad when it's your arms, hands, and legs. Terrifying when it involves the ability to swallow and breathe. That is a challenging part of this journey; seeing parents and families grief-stricken as their cherished little one has moved on from this life to the next. And that is hard. Very hard. It simply isn't fair. Watching little ones struggle with overwhelming physical challenges and knowing that the best scenario is a life dependent upon others for basic necessities will be a fact of life. It simply isn't fair.
Not fair. Just like we ask why our little Emmett had to be born with this most challenging genetic disease, we have asked why many times. Why - the question that cannot be answered. Another question is why didn't everyone in the drug trial for Nusinersen get the real drug. Again, there isn't an easy answer. Sure, the question may scientifically be easy to answer. After all, it's a scientific study and that is what happens in studies ... but does that make it fair! But not when the drug is working yet other little ones were getting the placebo. Then it is a horrible reality! And obviously that happened. What we can say is that because of the kids (and their parents) around the globe involved in this trial and showing how successful it has been, this trial resulted in an earlier than anticipated wider release of the drug. No more placebo going out - the drug was simply too positive to be giving some little ones the placebo. Fantastic news. Of course, the next harsh reality will be getting the drug approved. And once approved what will it cost! Suggestions and speculation are that it will be extremely costly. What then?
Recently I was asked if it wasn't frustrating to be in a blind study when we didn't know if Emmett was getting the drug or not. If you've been reading from the beginning you know that initially, I (remember, I'm his grandpa) was against Emmett being in a drug study. I didn't want him to be put through the challenges and the unknown factors involved in the testing of a drug that was literally unknown. What kind of side effects? What would he have to endure? So many questions. I've said before, Alisha and Josh convinced us. Dr. Selby and her team convinced them. And the medical team at BC Childrens Hospital has been so amazing in their support. They are like family!
Talking about family, his team of night nurses are also family. The care and love that they provide Emmett is something for which we are all so thankful to have. They are there for the mundane things like diaper changes through to the extreme moments (fortunately these don't happen very often) of ensuring that his airway is open. They help him by turning and repositioning him - which he literally cannot do on his own (have you ever woken up in the middle of the night and felt you couldn't move? That's Emmett!) - and, when he wakes up at night they read to him, sing to him, and pat him on the back. Of course, they follow his monitors - the oximeter, the bi-pap, the food pump. Suction as needed and change sheets if he's drooled so much that his face is almost a puddle. This team of awesome individuals is critical to Emmett's care - and his parents' peace of mind.
Another reality is we do not know what will be next for Emmett. We are optimistic about him speaking and looking forward to that. He's very communicative with his chattering, his eyes, his facial expressions, and his hands. He knows a couple of sign language phrases but he cannot control his fingers enough for most sign language. He's getting more movement - his physiotherapist and occupational therapist have observed this. Yet we want so much more movement. Obviously with time and more medication and more prayer there will be more changes. Alisha and Josh constantly work with Emmett on all of his skills. It's a task that goes on almost every moment that Emmett is awake. Questions. Will he be able to take food by mouth again? He mimics us when we're eating but that is such a challenging thought. He has to be suctioned for saliva - he's not ready to swallow food! Will he continue to gain strength in his arms and legs. Will his grasp develop even further? Will he start talking? Only time will tell. We keep praying! And working.
Back to the question about if it was frustrating to be involved in a blind drug trial. When Emmett was diagnosed his strength was declining at a very drastic rate. It was beyond alarming as in September 2015 and early October 2015 we were all terrified of what faced this fragile little one so when the only potential hope offered was an experimental drug study without knowing if he would get the drug or not - or if the drug would even work - with assurances from Dr. Selby that they, Alisha and Josh, were in the position of control with Emmett's procedures and could withdraw if they felt it was too challenging for him, they literally jumped for the opportunity. Parents do everything for their kids - that's just how it is. Emmett was stable but very weak when he began the trial. Between 4 and 6 months he lost a lot of movement. With the trial injections, we saw him showing signs of improvement. However, we also saw his respiratory system continue to drop over the first few weeks of the treatment. Small signs of improvement yet also signs that the disease was still taking more away from his tiny body. Yet, we were witnessing a miracle and while no one could tell us that Emmett was on the actual drug that first year, as weeks turned to months we knew. (In fact, as Emmett is now in the second year of the study where we know he is getting the drug - we are still blind about the first year. We assume he was, but as the study Emmett is participating in has been extended we still do not know for certain about year one.) Improvement is an amazing adjective for a genetic disease that usually only gets to use adjectives like decline and, hopefully, stabilized. Up until now, improvement has simply not been used with little ones dealt the genetic cards that have given them Spinal Muscular Atrophy Type 1 (or Type 2, 3, or 4). SMA is the Number 1 genetic cause of death for a reason. It takes beautiful little babies away from their families. As our family saw Emmett improving, we had to deal with the grief and difficulty that other families were having as their little ones lost the battle with SMA. Words do not describe this difficult challenge. We simply thank them for supporting us as we sought to support them.
Unfortunately, that is the harsh reality of a drug trial. All of the parents knew the risk. All knew that their little ones had a significantly high risk of death without the trial - and this trial has now already resulted in many children now getting the drug. In fact, at least three older kids that Emmett has seen at Canuck Place will be getting Nusinersen, now called Spinraza, starting early in the new year. This is exciting. There is still much sadness, however, as in our FaceBook extended SMA family we read of little ones' deaths every week. Parents contacting us about the drug - some able to access it but others not. Excitement on one hand for friends getting the drug and sorrow for those do not. And then there are Emmett's friends who were in the trial but did not survive. Emmett didn't get to know them - none of them were strong enough for playdates, Emmett included - and that is so very sad. But at one time or another Emmett was at BC Childrens Hospital at the same time as they were. I don't recall if all four were ever there all at the same time - but I think it happened. We will tell Emmett about these friends of his - close friends that he never got to have over for playdates. The twin sister's Emma and Addison and the little boy from the Okanagan, Atticus! Obviously, we don't know any of the details - if it was because they got the placebo or if there were other complications. But we celebrate their lives and the fact that they helped bring this drug to the world. When we talk with Emmett about these three little angels, we will marvel about the similarities in their names. We will likely share some tears too.
Emma, Addison, and Atticus! You are heroes and we miss you. All of us supporting Emmett are thinking of each of you and your families.
We must cure SMA!
Milestones! Each month is a milestone. And December 9 is 21 months for Emmett! We are so excited! And tired. As we have been on this journey with Emmett, we have realized that the specialists' initial statements that we may not ever see Emmett in pre-school, hear him talk, or live much past the age of two were the general understanding of youngsters with Spinal Muscular Atrophy Type 1. And while very fact based are not going to be the outcome for our little Emmett. He is powering his way through life - albeit with muscles that aren't very powerful. But his antics and expressions make up for his lack of movement.
You may have heard that the weather in BC has been rather rainy. I think the weather report was two days each in October and November with no rain. Balance - rain! So when it was sunny out then Emmett and Alisha had to get outside and play. Walks take quite some time but they're worth it.This was a local park that is fun to visit.
Emmett's Christmas tree is up with lights and decorations on and his house gets lit up at night with some artistic light displays (set up by both grandpa's) that he enjoys admiring. Emmett does not tire of decorating the tree. Literally. He has multiple times had the ornaments removed from the tree so that he can look at them up close, one at a time, before they go back on the tree. He is fascinated by them. So much so that we wonder if he will allow the tree to come down after Christmas!
Emmett, Alisha, Josh, and Rhonda went to Canuck Place in Abbotsford where they got to get up close to a bunch of reptiles. Emmett really wasn't sure about them. If you're wondering what Rhonda is laughing about, let me just say that she does not own a snakeskin scarf! Brave woman!
Emmett has recently discovered he is more into warm blooded creatures. His early Christmas present (or is it his daddy's?) is a seven month old cat that they've named Wendell. After a couple of hours this creature changed from a scaredy cat into a purring machine - Wendell is absolutely at home. While he is still getting used to Emmett, Emmett absolutely adores his kitty cat. They are going to be great buddies. I love the expression on Emmett's face the moment Wendell jumped from beside him to the back of the sofa. Wonderment!
What an amazing time of year. Emmett is 21 months old! Lots of laughs and giggles from the Willms house to yours. (Oh, and Emmett didn't get a new balloon this month either. But Ella the Elephant did get a refill! Here are a few more videos of Emmett ... if you have time!
Emmett was ready for his nap as he headed back into the house from a long walk/roll. As such he exhibits characteristics of a tired driver!